PMID- 36058861
OWN - NLM
STAT- MEDLINE
DCOM- 20220907
LR  - 20220907
IS  - 0485-1439 (Print)
IS  - 0485-1439 (Linking)
VI  - 63
IP  - 8
DP  - 2022
TI  - [Clinical significance of detecting human leukocyte antigen class I 
      allele-lacking leukocytes in aplastic anemia].
PG  - 899-905
LID - 10.11406/rinketsu.63.899 [doi]
AB  - Human leukocyte antigen (HLA) class I allele-lacking [HLA (-)] leukocytes provide 
      compelling evidence that cytotoxic T-lymphocytes are involved in the development 
      of aplastic anemia (AA). However, the clinical significance and precise 
      mechanisms underlying clonal hematopoiesis by HLA (-) hematopoietic stem 
      progenitor cells remain unknown. In HLA (-) cells from patients with AA, we 
      discovered a common nonsense mutation at codon19 (c.19C>T, p.R7X) in exon1 
      (Exon1(mut)) of different HLA-A and HLA-B alleles. Exon1 mutation detection using 
      droplet digital polymerase chain reaction (ddPCR) is a powerful tool for 
      diagnosing immune pathophysiology in patients with bone marrow failure. We also 
      looked at the prognosis of 633 patients with AA, including 127 with HLA (-) 
      leukocytes who had been followed up for a long time. In Japanese patients with 
      AA, HLA (-) leukocytes and concomitant aberrant clones were not associated with 
      clonal evolution to MDS/AML. In patients with AA and both marker (-) leukocytes, 
      HLA (-) leukocytes may indicate a lower risk of developing secondary paroxysmal 
      nocturnal hemoglobinuria (PNH). Detecting HLA (-) leukocytes is critical for 
      managing patients with AA and assisting physicians in selecting appropriate 
      therapy.
FAU - Hosokawa, Kohei
AU  - Hosokawa K
AD  - Department of Hematology, Faculty of Medicine, Institute of Medical 
      Pharmaceutical and Health Sciences, Kanazawa University.
FAU - Nakao, Shinji
AU  - Nakao S
AD  - Department of Hematology, Faculty of Medicine, Institute of Medical 
      Pharmaceutical and Health Sciences, Kanazawa University.
AD  - Japanese Red Cross Ishikawa Blood Center.
LA  - jpn
PT  - Journal Article
PL  - Japan
TA  - Rinsho Ketsueki
JT  - [Rinsho ketsueki] The Japanese journal of clinical hematology
JID - 2984782R
RN  - 0 (HLA Antigens)
RN  - 0 (Histocompatibility Antigens Class I)
SB  - IM
MH  - Alleles
MH  - *Anemia, Aplastic/therapy
MH  - HLA Antigens
MH  - *Hemoglobinuria, Paroxysmal/complications/genetics
MH  - Histocompatibility Antigens Class I/genetics
MH  - Humans
OTO - NOTNLM
OT  - Aplastic anemia
OT  - Exon1
OT  - Human leukocyte antigen class I allele-lacking leukocytes
EDAT- 2022/09/05 06:00
MHDA- 2022/09/08 06:00
CRDT- 2022/09/04 22:23
PHST- 2022/09/04 22:23 [entrez]
PHST- 2022/09/05 06:00 [pubmed]
PHST- 2022/09/08 06:00 [medline]
AID - 10.11406/rinketsu.63.899 [doi]
PST - ppublish
SO  - Rinsho Ketsueki. 2022;63(8):899-905. doi: 10.11406/rinketsu.63.899.